Affordable Access

Continuous muscle activity, Morvan's syndrome and limbic encephalitis: ionic or non ionic disorders?

Authors
  • Serratrice, G1
  • Serratrice, J
  • 1 CHU Timone, Université de la Méditerranée, Marseille, France. [email protected] , (France)
Type
Published Article
Journal
Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology
Publication Date
Jun 01, 2011
Volume
30
Issue
1
Pages
32–33
Identifiers
PMID: 21842591
Source
Medline
License
Unknown

Abstract

The early pathophysiologic study showed increasing evidence that autoimmunity is implicated in the pathogenesis of neuromyotonia. Antibodies to voltage gated potassium channel were detected in the serum of patients who had peripheral nerves hyperexcitability and also Morvan's disease or limbic encephalitis. These discoveries offered new approaches to treatments. Recently, antibodies previously attributed to VGKC recognise 2 surface antigens LGI1 and CASPR2 into the VGKC complex. Finally, VGKC antibodies are directed to 2 proteins the first one is a key hippocampic protein containing pre and post synaptic proteins. The second one CASPR2 is an hippocampic and paranodal protein. There clinical significance is different: hyperexcitability, limbic encephalitis without thymoma for LGI1, hyperexcitability, Morvan limbic encephalitis and frequent thymoma for CASPR2. In conclusion, the term NMT--LE--VGKC should be changed to NMT--LE with LGII and CASPR2 antibodies and classified as auto immune synaptic disorders. Mutations in genes encoding both these proteins are found in hereditary epilepsy and other syndromes. Various potassium channelopathies are closely linked to Morvan's syndromes. A new classification of antibodies will be necessary.

Report this publication

Statistics

Seen <100 times